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      Cardiac Involvement in Werdnig-Hoffmann’s Spinal Muscular At rophy


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          Despite the increasing interest in cardiac involvement (CI) of neuromuscular disorders, only few data are available on CI in spinal muscular atrophy (SMA). We tried to determine the cardiac manifestations of SMA, their incidence rates and the necessity of cardiac therapy in patients with SMA and CI. Eight patients with SMA, aged 10–79 years, underwent clinical cardiologic examination, ECG, echocardiography and ambulatory ECG. The most frequent findings were angina pectoris, palpitations, exertional dyspnea, ST abnormalities, couplets, thickened myocardium and diastolic dysfunction. In conclusion, cardiac manifestations of SMA mainly comprise ECG abnormalities and thickened myocardium. CI in SMA frequently necessitates cardiac therapy.

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          Most cited references1

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          Progression of Cardiac Involvement in Patients with Myotonic Dystrophy, Becker’s Muscular Dystrophy and Mitochondrial Myopathy during a 2-Year Follow-Up

          The aim of this prospective noninvasive follow-up study was (1) to assess the progression of cardiac involvement (CI) in patients with myotonic dystrophy (MD), Becker’s muscular dystrophy (BMD) and mitochondrial myopathy (MMP), (2) to find out if CI and neurologic impairment are related and (3) to determine how often cardiac investigations should be performed. Clinical, electrocardiographic, echocardiographic and 24-hour ambulatory electrocardiographic examinations were performed at yearly intervals. CI was assessed qualitatively by the presence of pathologic examinations and quantitatively by the number of pathologic examinations. Qualitatively, CI was present at baseline in 36 of the 38 cases (18–68 years) and the number of cases did not change. Quantitatively, CI progressed after 1 (2) year in 50% (50%) of patients with MD (n = 16), 0% (43%) of patients with BMD (n = 7) and 27% (27%) of patients with MMP (n = 15). CI and neurologic impairment were not related (p = 0.54). Cardiac investigations should be repeated at yearly intervals irrespective of whether there is neurologic deterioration or not.

            Author and article information

            S. Karger AG
            February 2000
            23 March 2000
            : 92
            : 3
            : 178-182
            aLudwig Boltzmann Institute for Epilepsy and Neuromuscular Disorders, and bZweite Medizinische Abteilung, Krankenanstalt Rudolfstiftung, Vienna, Austria
            6968 Cardiology 1999;92:178–182
            © 2000 S. Karger AG, Basel

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            Page count
            Figures: 1, Tables: 2, References: 19, Pages: 5
            Congenital Heart Disease

            General medicine,Neurology,Cardiovascular Medicine,Internal medicine,Nephrology
            Cardiac abnormalities,Neuromuscular disorders,Werdnig-Hoffmann’s atrophy,Neuronopathy,Echocardiography


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